Table 3 Clinical presentation, laboratory data, treatment and outcome of CNO patients cohort divided following presence or absence of FBLIM1 variant. § 9 patients were lost at last follow-up
Patients without FBLIM1 variants (62 patients) | Patients with FBLIM1 variants (18 patients) | P | |
|---|---|---|---|
Gender female | 41 (66%) | 11 (61%) | 0,78 |
Median Age at onset | 9.5 y (range 1.5–16) | 8.9 y (range 1,5–12) | 0,97 |
Clinical data | |||
Fever | 30 (48%) | 4 (22%) | 0,06 |
Swelling | 11 (18%) | 7 (38%) | 0,1 |
Pain | 60 (96%) | 15 (83%) | 0,07 |
Sites | 8.9 (median) | ||
Long bones | 46 (74%) | 13 (72%) | 1,0 |
Pelvis | 23 (37%) | 11 (61%) | 0,1 |
Column | 22 (35%) | 5 (27%) | 0,78 |
Clavicle | 16 (26%) | 5 (27%) | 1,0 |
Chest and ribs | 24 (39%) | 3 (16%) | 0,09 |
Foot and hands | 15 (24%) | 4 (22%) | 1,0 |
Mandible | 11 (18%) | 1 (5%) | 0,28 |
Skulls | 3 (5%) | 0 (0%) | 1,0 |
Skin involvements | 11 (18%) | 4 (22%) | 0,73 |
Bowel involvements | 3 (5%) | 1 (6%) | 1,0 |
Renal involvements | 2 (3%) | 0 (0%) | 1,0 |
Laboratory data | |||
Elevated CRP | 32 (52%) | 14 (77%) | 0,06 |
Elevated ESR | 41 (66%) | 13 (72%) | 0,78 |
Treatment | |||
NSAIDs | 55 (89%) | 17 (94%) | 0,67 |
Steroids | 20 (32%) | 6 (33%) | 1,0 |
MTX | 12 (19%) | 3 (16%) | 1,0 |
Sulfasalazine | 10 (16%) | 3 (16%) | 1,0 |
Adalimumab | 3 (5%) | 1 (5%) | 1,0 |
Etanercept | 8 (13%) | 2 (11%) | 1,0 |
Infliximab | 3 (5%) | 2 (11%) | 0,31 |
Anakinra | 9 (15%) | 3 (17%) | 1,0 |
Neridronate | 2 (3%) | 1 (6%) | 1,0 |
Pamidronate | 29 (47%) | 8 (44%) | 1,0 |
Outcome at last follow up§ | |||
Remission without therapy | 32 (52%) | 5 (28%) | 0,11 |
Remission on therapy | 18 (29%) | 5 (28%) | 1,0 |
Active disease | 6 (10%) | 5 (28%) | 0,11 |